Words similar to fibrillin
Example sentences for: fibrillin
How can you use “fibrillin” in a sentence? Here are some example sentences to help you improve your vocabulary:
In Case 2 with del exons 44-46, fibrillin synthesis was normal (109% of control), indicating that the mutant fibrillin molecules are stable.
The deposition of newly synthesized fibrillin into the matrix was greatly reduced (13 % of normal), however, suggesting that the internally deleted mutant fibrillin-1 molecules are secreted and exert a dominant-negative effect on extracellular microfibril assembly.
Primary fibroblast cultures from patients and control individuals were grown to confluency and then metabolically labeled with 35S-cysteine as described [ 17 18 ] . The soluble cell lysis fraction, containing newly synthesized labeled fibrillin-1 molecules, and the insoluble fraction, representing the labeled fibrillin in the extracellular matrix, were fractionated by SDS polyacrylamide gel electrophoresis and autoradiographed.
3. In addition, we found that certain cysteine substitutions in EGF-like domains and located immediately N-terminal of LTBP-like domains, cause protein instability and greatly reduced matrix deposition [ 9 ] . Taken together, our data support a model in which misfolding or partial deletion of LTBP-like domains results in proteolytic cleavage of the mutant fibrillin molecules.
This hypothesis is not only based on analysis of Case 1 reported here but on a similar analysis of five previously reported cases, one with exon 51 skipping [ 19 ] and four with cysteine substitutions immediately N-terminal of the LTBP like domains [ 9 ] . It is also possible, however, that proteolytic fragments of the mutant protein associate with the normal length fibrillin molecules and inhibit their secretion.
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